DMD monoclonal antibody, clone AOGG-4
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Specification
Product Description
Rabbit monoclonal antibody raised against synthetic peptide of human DMD.
Immunogen
A synthetic peptide corresponding to human DMD.
Host
Rabbit
Theoretical MW (kDa)
426.75
Reactivity
Human
Form
Liquid
Purification
Affinity purification
Isotype
IgG
Recommend Usage
Western Blot (1:500-1:2000)
The optimal working dilution should be determined by the end user.Storage Buffer
In PBS, 150 mM NaCl, pH 7.4 (50% glycerol, 0.02% sodium azide).
Storage Instruction
Store at -20°C for one year. After reconstitution, at 4°C for one month. It can also be aliquotted and stored frozen at -20°C for a longer time. Avoid repeated freezing and thawing.
Note
This product contains sodium azide: a POISONOUS AND HAZARDOUS SUBSTANCE which should be handled by trained staff only.
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Applications
Western Blot (Tissue lysate)
Western Blot analysis of human fetal heart tissue lysate with DMD monoclonal antibody, clone AOGG-4 (Cat # MAB19996). -
Gene Info — DMD
Entrez GeneID
1756Protein Accession#
P11532Gene Name
DMD
Gene Alias
BMD, CMD3B, DXS142, DXS164, DXS206, DXS230, DXS239, DXS268, DXS269, DXS270, DXS272
Gene Description
dystrophin
Gene Ontology
HyperlinkGene Summary
The dystrophin gene is the largest gene found in nature, measuring 2.4 Mb. The gene was identified through a positional cloning approach, targeted at the isolation of the gene responsible for Duchenne (DMD) and Becker (BMD) Muscular Dystrophies. DMD is a recessive, fatal, X-linked disorder occurring at a frequency of about 1 in 3,500 new-born males. BMD is a milder allelic form. In general, DMD patients carry mutations which cause premature translation termination (nonsense or frame shift mutations), while in BMD patients dystrophin is reduced either in molecular weight (derived from in-frame deletions) or in expression level. The dystrophin gene is highly complex, containing at least eight independent, tissue-specific promoters and two polyA-addition sites. Furthermore, dystrophin RNA is differentially spliced, producing a range of different transcripts, encoding a large set of protein isoforms. Dystrophin (as encoded by the Dp427 transcripts) is a large, rod-like cytoskeletal protein which is found at the inner surface of muscle fibers. Dystrophin is part of the dystrophin-glycoprotein complex (DGC), which bridges the inner cytoskeleton (F-actin) and the extra-cellular matrix. [provided by RefSeq
Other Designations
Duchenne muscular dystrophy protein|OTTHUMP00000023117|OTTHUMP00000023124|OTTHUMP00000023125|OTTHUMP00000023126|muscular dystrophy, Duchenne and Becker types
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Interactome
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Pathway
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Disease
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